Desmoid tumor in puerperium period: a case report.

Desmoid tumors (DTs) are rare tumors classified as deep fibromatoses taking origin from fascial or musculoaponeurotic structures. With pregnancy and surgical scars considered in the etiology, most anterior abdominal wall DTs occur in women in their reproductive years, especially during a pregnancy or post-partum. Herein, we present development of DT in a female patient in the post-partum period following cesarean delivery, which manifested itself with a growing mass in anterior abdominal wall. In our case, possibility of hematoma most probably located beneath the fascia was considered initially as a complication of cesarean section based on ultrasonographic examination and location of the lesion, while upon lack of either spontaneous regression with eventual diminish in size or resolve of symptoms within six weeks, further investigation via MRI and tru-cut biopsy revealed the diagnosis of abdominal DT. Radical tumor extirpation with resection of an adequate margin of surrounding normal tissue was applied, and the post-operative period was uneventful.

Aggressive (deep or desmoid-type) fibromatoses are locally infiltrative collagen-forming tumours with potential for recurrence but not metastasis. They exert their clinical effects primarily in relation to location and have variable biological behaviour. In sporadic cases there are somatic mutations in the β-catenin (CTNNB1) gene on 3p21, resulting in immunohistochemically demonstrable overexpression in nuclei. Fibromatosis in patients with familial adenomatous polyposis (FAP) harbours inactivating germline mutations in the desmoid region of the adenomatous polyposis coli (APC) gene on 5q21-q22. The primary management is surgical, though some desmoids cease to grow and can be watched.

To review our experience regarding the difference in management and treatment outcomes of aggressive fibromatosis of the head and neck region in children and adults, emphasizing, in particular, the role of conservative surgery in comprehensive treatment strategies.

The purpose of this study was to evaluate the impact of prophylactic laparoscopic colectomy on the risk of developing DT in patients with FAP. Patients with classical FAP with defined genotype who underwent either open or laparoscopic colectomy between 1947 and 2011 were included in the study. Laparoscopic surgery decreased the risk of DT after prophylactic colectomy in patients with FAP.

Desmoid fibromatosis (DF) carries a significant morbidity and a recognised mortality. Despite this there are currently limited diagnostic or treatment algorithms specific to cases of extra-abdominal DF. Historically surgical excision has formed the cornerstone of treatment. Recently however a paradigm shift has meant many practitioners now adopt a more conservative approach, placing emphasis on active surveillance, function preserving resections, and non-surgical oncologic therapies.

Desmoid tumor, or aggressive fibromatosis, is a rare, histologically benign, fibroblastic lesion that infrequently presents in the head and neck. The most common treatment is surgery, but desmoid tumors are characteristically associated with a high local recurrence rate after resection. The efficacy of postoperative radiotherapy is controversial. Its potential benefit should be carefully balanced against possible radiation-induced adverse effects. Alternative treatment modalities, such as primary radiotherapy and medical treatment or a wait-and-see policy, may be preferable to mutilating surgery.